| 摘要 |
Desmoid tumor (aggressive fibromatosis) is a benign neoplasm with a high rate of recurrence, but without metastasis, it has been reported as being one of the most common histological subtypes of chest wall soft tissue sarcoma. Risk factors for desmoid tumor, such as prior surgery, age at desmoid tumor diagnosis, pregnancy, and family fistory were sought. The literature review indicated that desmoid tumor are exceedingly common in familial adenomatous polyposis (FAP) where the comparative risk is 852 times that of the general population. A germ-line mutation of the adenomatous polyposis coli (APC) gene and overexpression of betacatenin are involved in the development of the desmoid tumor in FAP. These tumors are chiefly found in the intra-abdominal cavity and abdominal wall but rarely in the chest wall. We present a case of a 21-year-old young man. He was admitted with a protruding anterior chest wall mass which he had for 5 years. He also had a history of FAP and underwent a colectomy 8 years ago. Resevtion of the tumor with chest wall reconstruction was performed. No adjuvant postoperative therapy was done. Twelve monyhs after the operation, there was no evidence of recurrence. The main treatments which include pharmacological therapy, hormonal therapy, radiation therapy, chemotherapy have all been reported. The surgical treatment with aggressive wide excision is the main choice of therapy. Because of the high recurrence rate of desmoid tumors, we intend to follow up this patient for a long period. |
